Diagnostic Testing

Clinical features – appearance of lesions, erosions and blisters, Nikolsky sign, presence or absence of scarring and distribution of lesions.

Biopsy of skin or mucous membrane, with characteristic histological features.

Direct and indirect immunofluorescence – detection of autoantibodies either in a biopsy specimen (direct) or in the patient’s serum (indirect). Indirect (circulating) antibodies are helpful in confirming the diagnosis. The enzyme-linked immunoabsorbent assay (ELISA) is helpful for diagnosis, particularly if the immunofluorescence studies are negative, and to differentiate PV from PF; in the future newer more specific ELISAs hold promise as a prognostic indicator.

The following investigations are also recommended during disease assessment and management:

Full blood count and differential

Blood urea and electrolytes

Liver function tests

Blood glucose

Antinuclear antibody (differential of pemphigus erythematosus)

Thiopurine methyltransferase (TPMT) levels (if azathioprine is to be used)

Chest X-ray

Urinalysis

Blood pressure

Bone density scan (early in the course of treatment is recommended, and repeated periodically)

About IPPF

The International Pemphigus & Pemphigoid Foundation’s most important objectives are to provide patients and doctors worldwide with information about pemphigus and pemphigoid, and to provide patients and their caregivers much needed comfort and support so they can continue to live active, productive lives.Read more »